[Familial testicular regression syndrome]

The rare syndrome of embryonic testicular regression or agonadism with XY karyotype is described in this paper in a sibship of 7 including 3 cases of regression at a earlier time (in three amenorrheic sisters and on case of tardive and incomplete regression in a brother with bilateral testicular hypotrophy. The antigen H-Y is present in the three sisters. The pathogenesis of this syndrome, compared with that of pure gonadal dysgenesis is discussed.